Efficacy of Noninvasive Ventilation in Amyotrophic Lateral Sclerosis (ALS)
| Status: | Completed |
|---|---|
| Conditions: | Neurology |
| Therapuetic Areas: | Neurology |
| Healthy: | No |
| Age Range: | 18 - 80 |
| Updated: | 4/21/2016 |
| Start Date: | May 2007 |
| End Date: | June 2011 |
Prevalence and Severity of Nocturnal Oxygenation and Ventilation Failure in Patients With Amyotrophic Lateral Sclerosis Using Noninvasive Ventilation
This study will test the hypothesis that noninvasive ventilation (NIV) as prescribed in
current medical practice for use in amyotrophic Lateral Sclerosis (ALS) patients fails to
deliver adequate breathing support over a night of use in the patient's home. ALS patients
who come to the ALS Center for their routine 3 month follow up exam and are currently using
NIV will be asked to complete questionnaires regarding their quality of sleep, quality of
life and general level of function, and to undergo a home sleep study, using a safe,
comfortable and reliable breathing monitoring system during a night of sleep. If the
questionnaires or the sleep study show failure of the breathing device, the investigators
will work with the patient to fix the problem and then offer a second study to make sure
that the changes were helpful. The results of this study may help to develop subsequent
studies and to improve the guidelines used for care of ALS patients.
current medical practice for use in amyotrophic Lateral Sclerosis (ALS) patients fails to
deliver adequate breathing support over a night of use in the patient's home. ALS patients
who come to the ALS Center for their routine 3 month follow up exam and are currently using
NIV will be asked to complete questionnaires regarding their quality of sleep, quality of
life and general level of function, and to undergo a home sleep study, using a safe,
comfortable and reliable breathing monitoring system during a night of sleep. If the
questionnaires or the sleep study show failure of the breathing device, the investigators
will work with the patient to fix the problem and then offer a second study to make sure
that the changes were helpful. The results of this study may help to develop subsequent
studies and to improve the guidelines used for care of ALS patients.
Amyotrophic Lateral Sclerosis (ALS), also known as "Lou Gehrig's Disease", is a fatal
disorder that causes breathing failure due to progressive weakness of the muscles of
breathing. Breathing assist devices known as noninvasive ventilation (NIV) are offered to
ALS patients when their breathing function worsens. These devices deliver breathing
assistance via a mask on the nose or nose and mouth, and are thought to be particularly
important to be used during sleep, when breathing often becomes more shallow and irregular.
However, although these devices have become the standard of therapy in ALS patients once
their lung function worsens, it remains unclear how effective these devices actually are
when a patient is sleeping, partly because of the practical difficulties in applying the
device properly and keeping it applied throughout the sleep period, and partly because they
are most commonly prescribed without objective evidence regarding how much breathing support
the patient needs as the disease progresses and the breathing muscles weaken further.
disorder that causes breathing failure due to progressive weakness of the muscles of
breathing. Breathing assist devices known as noninvasive ventilation (NIV) are offered to
ALS patients when their breathing function worsens. These devices deliver breathing
assistance via a mask on the nose or nose and mouth, and are thought to be particularly
important to be used during sleep, when breathing often becomes more shallow and irregular.
However, although these devices have become the standard of therapy in ALS patients once
their lung function worsens, it remains unclear how effective these devices actually are
when a patient is sleeping, partly because of the practical difficulties in applying the
device properly and keeping it applied throughout the sleep period, and partly because they
are most commonly prescribed without objective evidence regarding how much breathing support
the patient needs as the disease progresses and the breathing muscles weaken further.
Inclusion Criteria:
- clinical diagnosis of amyotrophic lateral sclerosis (El Escorial criteria)
- age 18 to 18 years old
- using nocturnal noninvasive ventilation to treat respiratory insufficiency at least 4
hours per night, for at least 4 nights per week
Exclusion Criteria:
- inability to safely use NIPPV because of bulbar dysfunction
- indications for tracheostomy assisted ventilation due to inability to clear
secretions from the airway
- presence of comorbid conditions with a life expectancy < 6 months
- presence of advanced dementia
- unwillingness to follow up at the Eleanor and Lou Gehrig ALS/MDA Center at Columbia
University on a regular basis
- previously diagnosed obstructive sleep apnea
- residence outside the New York metropolitan area.
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